Dermatologic Manifestations of Staphylococcal Scalded Skin Syndrome Clinical Presentation
- Author: Paul Benson, MD; Chief Editor: Dirk M Elston, MD more...
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- Staphylococcal scalded skin syndrome (SSSS) originates from a focus of infection that may be a purulent conjunctivitis, otitis media, or occult nasopharyngeal infection. A case of staphylococcal scalded skin syndrome from a nasal septal abscess, presumably arising from a traumatic hematoma (a tsunami survivor), has been reported. In premature infants, a previous amniocentesis procedure and intrauterine infection have been reported as triggers for staphylococcal scalded skin syndrome.[19, 20]
- Staphylococcal scalded skin syndrome usually begins with fever, irritability, and a generalized, faint, orange-red, macular erythema with cutaneous tenderness.
- Periorificial and flexural accentuation may be observed. A positive Nikolsky sign (slippage of the superficial layer of the epithelium on gentle pressure) can often be elicited at this stage.
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- Subsequent generalized involvement occurs elsewhere on the body, but infection spares the mucous membranes.
- As sheets of epidermis are shed, a moist erythematous base is revealed.
- Despite the dramatic clinical picture, the entire process usually subsides with superficial desquamation, and healing is usually complete within 5-7 days.
- In adults, it is frequently followed by bacteremia and pneumonia, favoring a poor prognosis.
- Cultures obtained from intact bullae are usually sterile; this finding is consistent with hematogenous dissemination of a toxin produced at a distant focus of staphylococcal infection.
- An abortive form of staphylococcal scalded skin syndrome, known as the scarlatiniform variant, shows the early erythrodermic and final desquamative stages seen in staphylococcal scalded skin syndrome, but the bullous stage does not occur.
- Other intermediate forms of scalded skin syndrome begin as localized bullous impetigo, but they evolve to produce regionally limited bullae and denuded areas that may or may not harbor staphylococci.
- ET-A and ET-B are serine proteases that specifically target desmoglein-1. They are also sources of superantigenic activity and activate macrophages to produce proinflammatory cytokines such as tumor necrosis factor-alpha and interleukin 6. ET-A with an active site mutation was shown to possess a similar inflammatory activity, indicating that the inflammatory activity of ET-A was separate from its epidermolytic activity. Systemic symptoms, particularly the characteristic rash, are very likely a direct result of the toxin.
- A small number of patients with SSSS develop low titers of immunoglobulin G antibodies specific for desmoglein-1 after binding and systemic digestion of desmoglein-1 by staphylococcal exfoliative toxins. The relevance of this finding to the onset of autoimmune diseases remains to be proven. Other genetic or environmental factors may be needed to extend the immune response to encompass pathogenic antibodies and to produce overt clinical pemphigus foliaceus.
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